Atypical teratoid/rhabdoid tumor of the central nervous system: Clinicopathological features of two challenging cases

Introduction: Atypical Teratoid/Rhabdoid Tumor (AT/RT) is a rare aggressive neoplasm that mainly affects the pediatric population with a peak incidence in the first two years of life and a slight male predominance, whereas presentation of this neoplasm in older ages is extremely rare. Case presentation: Herein, we present two cases of AT/RT. In the first case, a 9 -year -old female presented with diplopia, dizziness, headache, and morning vomiting. CT Scan of the head demonstrated a heterogeneous mass in the left frontal -parietal region with vasogenic edema and midline deviation. In the second case, a 57 -year -old female presented with severe generalized headache, numbness, and tingling in the right hand. MRI revealed a lobulated cystic mass in the right occipitotemporal region, with surrounding edema compressing the left lateral ventricle and causing a midline shift to the left, and enlargement of the right lateral ventricle. In both case, histopathological and immunohistochemical examinations revealed the diagnosis of Atypical teratoid/Rhabdoid tumors. Clinical discussion: Microscopic examination demonstrated the proliferation of medium-sized to large cells with abundant eosinophilic cytoplasm, large vesicular eccentric nuclei, and conspicuous nucleoli with areas of necrosis and hemorrhage, thus confirming the diagnosis with adequate immunohistochemical staining. The first patient developed signs of recurrence and passed away six months later, whereas in the second case, the 57 -yearold female received radiotherapy for 6 weeks before being put on chemotherapy. Conclusion: Despite the challenges facing the diagnosis of this aggressive neoplasm, we managed to present our cases with detailed histopathological and immunohistochemical examinations.